Supporting adults who have Prader-Willi syndrome : caregivers' perspectives on the ethical and practical dilemmas : a thesis presented in partial fulfillment of the requirements for the degree of Doctor of Philosophy in Psychology at Massey University, Wellington, New Zealand
Prader-Willi Syndrome (PWS) is a complex genetic condition that typically results in
learning impairment and hyperphagia, which is the drive to consume excessive quantities of
food. Obesity often develops very rapidly and is life-threatening, presenting professional and
familial support people with a distinctive set of ethical and practical dilemmas. These unique
challenges can be characterised as a microcosm of a wider “safety versus autonomy” debate
within human services. Little is known about the nature or efficacy of publically funded
supports to adults who have PWS in Aotearoa/New Zealand. The aim of this thesis was to
investigate the ethos of current services and some of the outcomes associated with them. In
particular, it sought to illuminate the ways in which professional caregivers address the
challenges they face in their work, according to their own reported perceptions of their role.
The thesis begins with a review of the relevant international research literature, noting
the dominance of clinical experience and anecdotes over research studies within it. This
review has informed three investigations. First, a scoping study comprised of semi-structured
interviews was conducted with professional caregivers in workplace-based focus groups.
Second, an attempt was made to identify and survey all adults within Aotearoa/New Zealand
who have PWS regarding the supports they received. Finally, a thematic analysis of the
transcribed texts of interviews with individual caregivers was undertaken. These interviews
concerned the ways in which caregivers of adults with PWS experienced, conceptualised and
addressed the dilemmas inherent in their role.
Findings indicated that there are grounds for serious concern about both the wellbeing
of people with PWS in Aotearoa/New Zealand, and that of their caregivers. Significant levels
of obesity were reported in almost all service users with PWS, despite the ecological controls over access to calories imposed on them by caregivers. It seems likely that some adults with
PWS are at risk of serious harm while having their civil rights infringed to little or no effect.
Caregiver participants characterised their role in terms of the challenges it presented
them. The management of service users’ access to food was identified as only one of the
sources of such challenge. Challenging behaviour among people with PWS was reported to
generate the most significant and pressing difficulties. Caregivers reported responding to
these challenges by imposing substitute decisions, especially in relation to food, despite
awareness that such measures seemed to constitute a breach of ethical norms. They regarded
compulsory ecological controls as necessary for the service users’ safety, but also as a
compassionate response which prevented uncertainty in relation to food and hence spared
service users with PWS from subsequent anxiety.
Caregivers seemed in agreement with a view of client autonomy as relational and
seemed to employ a dialogical process featuring stories and responses to stories in collective
decision making. Despite the emphasis on individualism in contemporary services to people
with disabilities, interviewees often seemed to prioritise corporate wellbeing, even when
making decisions regarding access to food for a single individual with PWS.
The conclusions which may be drawn from these studies are limited by a number of
factors, notably small sample size. Nevertheless, they offer insights into the nature of the
caregiving task and the concept of autonomy within human services. Findings form the basis
of suggested innovations, both in caregiving practice and for future research.